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ONH hypoplasia - a subnormal number of optic nerve _____
axons
ONH hypoplasia

a nerve with hypoplasia will be small or large?
small
ONH hypoplasia will exist with a ______ ______ peripapillary halo with a ring of pigment outside the halo called the ____ ____ ____
yellowish mottled double ring sign
double ring sign

45% of patients with optic nerve hypoplasia have ____ malformations
CNS
what condition is associated with ONH hypoplasia
septo-optic dysplasia
what is the triad of septo-optic dysplasia?
ONH hypoplasia, midline brain abnormalities, pituitary gland dysfunction
onh hypoplasia warrants what two referrals?
neuroimaging and endocrinology
_____ _____ _____ ____ - congenital funnel shaped excavation of the posterior globe which involves the optic disc
morning glory disc anomaly
does morning glory affect more males or females?
females
along with an enlarged orange/pink funnel shaped area of excavation, what is a common finding with morning glory?
overlying central tuft of glial tissue
in patients with morning glory, retinal vessels run an abnormally ______ course over the peripapillary retina and branch at _____ angles
straight acute
is morning glory unilateral or bilateral?
unilateral
in cases of morning glory, SRF can accumulate leading to a...
serous macular detachment
morning glory

patients with morning glory will have an enlarged ____ ____
blind spot
morning glory has a strong association with some _____ _______ abnormalities
congenital brain
_____ _____ - outpouching of retina due to axial elongation from pathological myopia
posterior staphyloma
patients will have what VF defect at the staphyloma location?
scotoma
____ ___ ______ - an enlarged disc within a sharply demarcated white excavation inferiorly due to an incomplete closure of the embryonic fissure during gestation
optic disc coloboma
where does the coloboma occur?
inferiorly
coloboma may involve the adjacent _____ and _____
choroid retina
optic disc coloboma is associated with what three other findings?
serous RD, foveal hypoplasia, pigmented macular atrophy
pts with disc coloboma will have an enlarged ____ ____
blind spot
optic disc coloboma is associated with genetic disorders and mutations arising from _____ defects
midline
what are the CHARGE syndromes?
coloboma of the eye, heart defects, atresia, retardation of growth, ear abnormalities with deafness
coloboma

optic _____ - isolated excavation in the optic nerve head
pit
pits are most commonly on the ______/______ nerve head
inferior temporal
are optic disc pits unilateral or bilateral?
unilateral
30-75% of patients with an optic disc pit will develop ______
maculopathy
optic pits can cause _______-like degenerations of the _____ macula and shallow macular detachments
retinoschisis nasal
what are two treatments for optic pits?
laser photocoagulation and PPV
optic pit

optic pit OCT

patients with optic pits are generally asymptomatic unless _______ occurs
maculopathy
patients with optic disc pits will have an enlarged ____ ____
blind spot
are optic pits associated with any brain malformations?
no
megalopapilla is a disc that is larger than ____mm
2.5
patients with megalopapilla will have normal VA with an enlarged _____ _____
blind spot
is megalopapilla associated with any systemic conditions?
no
is congenital tilted disc syndrome normally unilateral or bilateral?
bilateral
in cases of congenital tilted disc syndrome:
superiotemporal disc is ________
inferionasal disc is displaced ______
elevated posteriorly
_____ ______ - retinal vessels emerge from the optic disc and initially course nasally before turning temporally, reversing to the normal pattern
situs inversus
in cases of congenital tilted disc syndrome, there may be a scleral crescent in what two places?
inferior or inferior nasal
tilted disc

choroidal and scleral crescents are usually _____ to the optic disc
temporal
choroidal and scleral crescents are due to an _____ insertion of optic nerve to globe
oblique
a scleral crescent occurs when _____ and _____ do not border ONH and there is visualization of the sclera
retina choroid
a choroid crescent occurs when the _____ does not border then ONH and there is better visualization of the choroid
retina
mal insertions

myelinated nerve fibers occur when myelination does not stop at the ____ ____ and appears in the retina
lamina cribosa
are myelinated nerve fibers usually unilateral or bilateral?
unilateral
myelinated NFL

if you suspect myelinated NFL, what are two DDx that need to be ruled out?
CWS and artery occlusion
myelinated NFL may create a ____ ____ VF defect
relative scotoma
_____ ______ - a benign glial remnant positioned on or over the ONH
bergmeisters papilla
hyaloid remnant

optic nerve _______ - a benign neoplasm over the ONH
melanocytoma
what is the mean age of diagnosis for ON melanocytoma?
50
are optic nerve melanocytoma more commonly unilateral or bilateral?
unilateral
optic nerve melanocytoma become more _____ over time
pigmented
optic nerve melanocytoma have a ___ risk of malignant transformation
low
if the optic nerve melanocytoma is greater than ____mm thick, there is a greater risk for malignant transformation
1.5
optic nerve melanocytoma

what are two hereditary conditions that cause ONH malformations
leber hereditary optic neuropathy and autosomal dominant optic atrophy
leber hereditary optic neuropathy arises from an inherited _____ mutation in mitochondrial DNA and manifests in young adulthood as a distinctive __________ optic neuropathy
point heredodegenerative
___% of males and ___% of females who carry LHON mutation manifest optic neuropathy
50 10
leber hereditary neuropathy is associated with ____ condition abnormalities
cardiac
typical lebers presentation:
young adult who develops acute painless severe _____ vision loss followed by similar vision loss in the fellow eye __-__ months later
unilateral 2 3
patients with lebers will have a ____ or ______ VF defect
central cecocentral
how with the optic nerve change with lebers?
hyperemia followed by pallor
lebers

autosomal dominant is the most ____ hereditary optic neuropathies
common
onset of autosomal dominant is within the ____ decade of life
first
vision is reduced _______ in both eyes in cases of autosomal dominant
symmetrically
autosomal dominant will cause disc pallor where?
temporal
autosomal dominant will cause what kind of visual field defect?
cecocentral
autosomal dominant

papilledema is unilateral or bilateral?
bilateral
papilledema is due to specifically what?
increased ICP
an increased CSF pressure leads to blocking of _______ transport of the axons that make up the ONH leading to disc edema
axoplasmic
papilledema may appear as an ________ extension of the ONH
anterior
papilledema may appear as edema of what retinal layer?
NFL
papilledema may show up as venous _______ or _______ and ________ vessels
congestion arcuate peripapillary
papilledema may come with peripapillary _____ hemes
splinter
papilledema

papilledema with patons lines

early papilledema:
disc _______
disc swelling
____ of disc margins
blurring of _____
hyperemia blurring NFL
fully developed papilledema:
gross ______ of the ONH
engorged veins
peripapillary _____ hemses
choroidal ______
retinal ______
elevation splinter folds striae
chronic papilledema:
____ hemes
less disc _______
_____ exudates
fewer hyperemia hard
LATE papilledema:
secondary optic ______
disc swelling subsides
_____ ONH
vessels become _____
___ vessels may form (collaterals)
atrophy gray narrow shunt
frisen scale grade 1:
____ _____ papilledema
___-shaped halo with a temporal gap
_____ margin blurring
normal temporal margin
___ SVP
very early C nasal negative
frisen scale grade 2:
_____ papilledema
circumferential halo
blurring of ____ borders
peripapillary halo significant ______ elevation
early all nasal
frisen scale grade 3:
_____ papilledema
obscuration of one or more major _____ ____ leaving the disc
______ diameter of ONH
_____ halo
moderate blood vessels increased ruffled
frisen scale grade 4:
______ papilledema
obscuration of ____ _____ on the disc
elevation of _____ ONH
may see some ____ and hemes
marked major vessels entire CWS
frisen scale grade 5:
____ papilledema
_____- shaped protrusion
obliteration of cup
obscuration of ____ vessels on and leaving the disc
severe dome all
headaches that are due to papilledema are often worse when _____ or _____ _____
laying bending down
transient vision obscuration due to papilledema occur as monocular or binocular blackouts which last for ___-____ seconds usually when patient ____ ______
3 4 changes posture